Scleroderma Renal Crısıs

Systemic sclerosis (SSc), also known as scleroderma, is a disease that can affect many tissue and organ systems. Contrary to expectations, SSc can also affect the kidneys frequently. Most renal involvements are in the form of asymptomatic proteinuria and elevated creatinine level. Scleroderma renal crisis (SRC), which is one of the most mortal clinical findings of SSc, is rarely seen. The diffuse skin involvement subtype of SSc, early stage of the disease (first 4 years), anti-RNA-polymerase III antibody positivity and corticosteroid use are risk factors for SRC. Angiotensin converting enzyme inhibitors (ACEi) are used in the treatment of SRC. For this reason, close follow-up of patients with high risk of SRC is recommended, as early initiation of treatment increases the chance of success. In the prophylactic use of ACEi, the prognosis may be worse since the clinical manifestations of SRC are suppressed, and the diagnosis of SRC is delayed and thus SRC treatment is delayed (ACEi are used in higher doses in treatment). Angiotensin receptor blockers and iloprost are alternatives to ACEi in the treatment of SRC. The decision for renal transplantation should not be rushed in patients treated for SRC, as renal function may return late.